混合性神经内分泌非神经内分泌肿瘤(MiNEN)是一种特殊的实体,可以发生在整个胃肠道性状,胰腺定位很少见。它们的主要特征是至少存在神经内分泌和上皮成分,每个占肿瘤质量的至少30%。上皮导管成分的存在定义了腺MiNEN。我们报告了一例59岁的女性,患有胰腺腺MiNEN,具有挑战性的诊断并成功治疗。还进行了系统的文献回顾和汇总分析,旨在确定胰腺腺MiNEN的治疗和结局。在190条确定的记录中,15项研究包括28例受胰腺-腺-MiNEN影响的患者纳入分析。胰腺腺MiNEN主要发生在男性(82.8%),平均年龄为61.7(范围:24-82)岁。仅在14.2%的病例中可以进行术前诊断。在介绍时,大多数患者已经患有晚期疾病(TNMIII期(53.8%)和IV期19.3%).55%的患者进行了辅助治疗,肿瘤复发率为30%。中位无病生存期(DFS)为12个月(范围:0-216个月),5年DFS为16.6%,而中位总生存期(OS)为12个月(范围:0-288个月),5年OS为23.5%。胰腺腺苷酸是罕见的,因为它们具有非常异质的行为,术前很少诊断,预后不良.局部MiNEN的治疗仍然依赖于根治性手术切除,这似乎对获得良好的肿瘤预后至关重要。MiNEN的国际注册对于提高对这种罕见肿瘤的认识并改善其结果是必要的。
Mixed neuroendocrine non-neuroendocrine neoplasms (
MiNEN) are a peculiar entity that can occur throughout the whole gastrointestinal trait, and pancreatic localization is rare. Their main characteristic is the presence of at least a neuroendocrine and an epithelial component, each accounting for at least 30% of the tumour mass. The presence of epithelial ductal component defines adeno-
MiNEN. We report a
case of a 59-year-old woman affected by pancreatic adeno-
MiNEN with challenging diagnosis and successfully treated. A systematic literature review and pooled analysis was also performed, aiming to define the management and outcomes of pancreatic adeno-MiNEN. Out of 190 identified records, 15 studies including 28 patients affected by pancreatic-adeno-
MiNEN were included in the analysis. Pancreatic adeno-
MiNEN occurred mainly in males (82.8%) and at a mean age of 61.7 (range: 24-82) years. Pre-operative diagnosis was possible only in 14.2% of cases. At presentation, the majority had already advanced disease (TNM stage III (53.8%) and stage IV 19.3%). Adjuvant therapy was performed in 55% of patients, and the tumour recurrence rate was in 30% of cases. Median disease-free survival (DFS) was 12 months (range: 0-216 months) with a 5-year DFS of 16.6%, while the median overall survival (OS) was 12 months (range: 0-288 months) with a 5-year OS of 23.5%. Pancreatic adeno-MiNENs are rare; as they have very heterogenous behaviour, they are rarely diagnosed preoperatively and have poor prognosis. Treatment of localised MiNEN still relies on radical surgical resection, which seems essential to achieve a good oncological prognosis. International registry on MiNEN is necessary to improve the knowledge on this rare tumour and to improve its outcomes.